This report summarizes congenital causes of pulmonary venous hypertension. Descriptions of both clinical and morphologic features of obstruction of congenital obstructions of the major pulmonary veins, including partial and total anamolous pulmonary venous connection, cor triatriatum, mitral valve atresia, supravalvular left atrial ring, and congenital mitral stenosis are illustrated. In contrast to acquired lesions which interfere with left ventricular filling, cogenital lesions are quite rare. This paper summarizes a large personal experience with the congenital lesions which lead to pulmonary venous hypertension.